Nephroblastoma nephroblastoma. Three associated congenital malformations 1. WAGR Wilms,aniridia, genital abnormalities, mental retardation (33% risk of tumor). http://pathophysiology.uams.edu/Spring01/peds/lect40-s01/tsld045.htm
Complete List Of MeSH-C Terms Found In References Related To Malignant Hyperthermia 2 Cleft Lip 2 Limb Deformities, congenital 2 Oral andEmbryonal 1 Anus Diseases 1 Aneurysm 1 nephroblastoma 1 Craniofacial http://www.bork.embl-heidelberg.de/g2d/list_mesh_disease.pl?U6589:Smith-Lemli-Op
Indian Pediatrics - Editorial The authors had reported various congenital anomalies of the central nervous tumorsoften associated with the BWS such as nephroblastoma, neuroblastoma, rhabdo http://www.indianpediatrics.net/mar2002/mar-299-304.htm
Extractions: L.S. Arya From the Division of Pediatric Oncology, Department of Pediatrics and *Rajendra Prasad Center for Ophthalmic Sciences, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110 029, India. Correspondence to: Dr. V. Thavaraj, Department of Pediatrics, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110 029, India. E-mail: sowmyam@mantraonline.com Manuscript received: May 31, 2001; Revision accepted: September 5, 2001. Beckwith-Wiedemann syndrome (BWS) was first recognized as a syndrome by Beckwith in 1963, when he reported autopsy findings on three unrelated children with omphalocele, macroglossia, cytomegaly of adrenal cortex, renal medullary hyperplasia and hyperplastic visceromegaly(1). In 1964 Wiedemann published an article on the same syndrome in three siblings with omphalocele, macrosmia and neonatal hypoglycemia(2). The incidence of BWS has been estimated to be 1:13700 live births(3). The increased risk of tumor formation in BWS patients is estimated to be 7.5%(4) and the risk is further increased to 10% if hemihypertrophy is present(4). It has been suggested that BWS can be inherited by the homozygous state of an autosomal recessive mutation(5). We describe a rare case report of partial expression of BWS associated with orbital rhabdomyosarcoma (RMS).
MEDLINEplus Medical Encyclopedia: Topics Beginning With N syndrome acute see Acute nephritic syndrome. nephroblastoma see Wilms Nephrogenicdiabetes insipidus - congenital see congenital nephrogenic diabetes insipidus; http://www.nlm.nih.gov/medlineplus/ency/encyclopedia_N.htm
Extractions: Other encyclopedia topics: A-Ag Ah-Ap Aq-Az B-Bk ... Z Na+ see Serum sodium Nabothian cyst Nail abnormalities Nail abnormalities - causes see Brittle nails - causes Nail blanch test see Capillary nail refill test Nail care for newborns Nail polish Nails - fungal infection see Fungal nail infection NALD see Adrenoleukodystrophy Nalfon see Fenoprofen calcium overdose Nalfon overdose Naphthalene Naprosyn overdose Naproxen sodium see Naprosyn overdose Narcissistic personality disorder Narcolepsy Narcotic abuse see Drug abuse Narcotics - screen see Toxicology screen Nasal allergies see Allergic rhinitis Nasal congestion Nasal CPAP Nasal discharge ... Nasal mucosal biopsy Nasal obstruction see Nasal congestion Nasal polyps Nasal septum repair see Septoplasty Nasal tumor see Juvenile angiofibroma Nasopharyngeal culture Natal teeth Natural short sleeper ... Nausea and vomiting Nausea in the morning - females see Morning sickness NBP see Prostatitis - nonbacterial NBT test see Nitroblue tetrazolium test NCV see Nerve conduction velocity Near drowning Nearsightedness Nearsightedness surgery see Corneal surgery Neatsfoot oil NEC see Necrotizing enterocolitis Neck lump Neck pain Neck stiffness see Neck pain Neck X-ray Neck/spinal injury see Spinal/neck injury Necrosis Necrosis - acute tubular see Acute tubular necrosis Necrosis - renal papillae see Renal papillary necrosis Necrosis - renal tubular see Acute tubular necrosis Necrotic see Necrosis Necrotizing colitis see Pseudomembranous colitis Necrotizing enterocolitis Necrotizing fasciitis see
Extractions: Up Tracheostomy Foreign bodies Epistaxis ... Rectal bleeding in childhood [ Childhood abdominal masses ] Choledochal cysts Cryptochidism Paediatric hernias Circumcision ... Neck lumps in children Liver Genitourinary Other Arises from neural crest tissue - usually adrenal medulla or sympathetic ganglia Show a range of malignancy from benign ganglioneuroma to malignant neuroblastoma Tumours in children are usually malignant 75% are abdominal 25% arise in thorax, pelvis or neck
Extractions: NORMAL SONOGRAPHIC ANATOMY Normal development of the anterior abdominal wall depends on the fusion of four ectomesodermic folds (cephalic, caudal and two lateral). At 810 weeks of gestation, all fetuses demonstrate herniation of the mid-gut that is visualized as a hyperechogenic mass in the base of the umbilical cord; retraction into the abdominal cavity occurs at 1012 weeks and is completed by 11 weeks and 5 days. The integrity of the abdominal wall should always be demonstrated; this can be achieved by transverse scans demonstrating the insertion of the umbilical cord. It is also important to visualize the urinary bladder within the fetal pelvis, because this rules out exstrophy of the bladder and of the cloaca. EXOMPHALOS Exomphalos results from failure of normal embryonic regression of the mid-gut from the umbilical stalk into the abdominal celom. The abdominal contents, including intestines and liver or spleen covered by a sac of parietal peritoneum and amnion, are herniated into the base of the umbilical cord.
Fetal Turmors - DIAGNOSIS OF CONGENITAL ABNORMALITIES - THE 18-23 WEEKS SCAN intrauterine exposure to antithyroid drugs or congenital metabolic disorders the mostfrequent renal tumor, while Wilms tumor (nephroblastoma) is extremely http://www.centrus.com.br/DiplomaFMF/SeriesFMF/18-23-weeks/chapter-11/tumors.htm
Extractions: Introduction Developmental errors during embryonic and fetal maturation may result in embryonic tumors. One hypothesis is that more cells are produced than are required for the formation of an organ or tissue and the origins of embryonic tumors rest in developmental errors in these surplus embryonic rudiments. Embryonic tumors developing after infancy are explained by the persistence of cell rests or developmental vestiges. Developmentally anomalous tissue (such as hamartomas and dysgenic gonads) is a source of neoplasms in older children and adults. When any of this developmentally abnormal tissue is present at birth, it is inferred that the cells failed to mature, migrate or differentiate properly during intrauterine life. Some neonatal and infantile tumors have a benign clinical behavior despite histological evidence of malignancy. Examples include congenital neuroblastomas and hepatoblastomas in the first year of life, and congenital and infantile fibromatosis, and sacrococcygeal teratomas in the first few months of life. The factors responsible for this oncogenic period of grace, which starts in utero and extends through the first few months of extrauterine life, are uncertain.
Ref6 Nucleolar organizer regions in congenital mesoblastic nephroma JFM, Krul EJ, MF T.Mesoblastic nephromas a study of 29 tumors from the SIOP nephroblastoma file http://www.anapath.necker.fr/site/SLMos/SLMos.data/Composants/cases/case06/Ref6.
Birth Disorder Information Directory - W Macules, Hereditary congenital Hypopigmented and Hyperpigmented) William('s)(Beuren) Syndrome List of Sites. Wilms Tumor (nephroblastoma) List of Sites; http://www.bdid.com/defectw.htm
Extractions: HOME W Syndrome Waaler Aarskog Syndrome (Hydrocephalus with Costovertebral Dysplasia and Sprengel Anomaly) Waardenburg Syndrome -Shah Syndrome with Hirschsprung Disease Wagner Syndrome (Erosive Vitreoretinopathy, Hyaloideoretinal Degeneration of Wagner, Wagner Vitreoretinal Degeneration) WAGR Syndrome Walbaum Titran Durieux Crepin Syndrome (Fibular Hypoplasia with Scapulo Pelvic Dysplasia and Absent 5th Fingers) Walker Dyson Syndrome (Aniridia-Mental Retardation Syndrome) Walker Warburg Syndrome (Cerebroocular Dysgenesis; Cerebroocular Dysplasia-Muscular Dystrophy Syndrome; Chemke Syndrome; COD-MD Syndrome; Hard +/- E Syndrome; H ydrocephalus
Www.georgetown.edu/dml/educ/path/lab19/lab19.txt This congenital hydronephrosis was secondary to an atresia of the urethra. 194a4.Wilms' Tumor - nephroblastoma History- This five year old boy was noted http://www.georgetown.edu/dml/educ/path/lab19/lab19.txt
Extractions: 31 [19-1a]: Pediatric Pathology 1. Congenital Cytomegalovirus History- This male infant was noted by ultrasound to have an enlarged abdomen at 24 weeks gestation. The 22- year-old mother gave a history of a brief febrile illness during her first few weeks of pregnancy. The infant was born by spontaneous vaginal delivery at 35 weeks gestation. The infant died shortly after birth of respiratory distress. A. At birth the infant was noted to have an enlarged abdomen with a prominent fluid wave that extended into his enlarged scrotum. Note the numerous petechiae (small hemorrhages) over the trunk, face and limbs. [19-1b]:1B. The abdomen was filled with clear yellow fluid that distended the abdomen, elevating the diaphragms and compressing the developing lungs. The liver was only mildly enlarged. [19-1c]:1C. The liver architecture is distorted by prominent lymphoid aggregates in and adjacent to portal areas. Bile ducts are lined by normal appearing or occasional enlarged epithelial cells. [19-1d]:1D. The enlarged bile duct epithelial cells are 3- 4X the size of the normal cells and contain an enlarged nucleus with a prominent eosinophilic inclusion surrounded by a clear "halo". These cells are pathognomonic of cytomegalovirus infection. [19-1e]:1E. The brain was mildly hydrocephalic with dilated ventricles and a thinned cerebral cortex. Upon sectioning the brain a coarseness was felt in the periventricular region representing areas of calcification. Questions 1. What is the incidence of CMV infection during pregnancy and how many infants will be mildly or severely infected? 2. What are the clinical manifestations of congenital CMV infection? 3. What other organisms are associated with congenital infections? [19-2a]:2. Congenital Group B Streptococcal Infection History- This term male infant was born 28 hours after premature rupture of the amniotic membranes. He was in mild respiratory distress at birth which progressed rapidly to death at 8 hours of age. A. The lungs at autopsy were consolidated and unevenly expanded. [19-2b]:2B. Bronchi, bronchioles and alveolar ducts are filled with acute inflammatory cells (neutrophils). [19-2c]:2C. Alveoli are distended by neutrophils and macrophages along with thin linear eosinophilic structures representing aspirated squamous cells (squames) that are sloughed from the infant's skin after about 25 weeks gestation. Note the faintly basophilic granular material in the alveolus representing clusters of bacteria. [19-2d]:2D. A section from the placenta of this infant displays marked subchorionic inflammation that extends through the chorion into the amnion (acute chorioamnionitis). Questions- 1. What are the routes through which an infant may become infected inutero? 2. How does Group B Streptococcal infection differ from a TORCH infection? 3. Describe the stages or degrees of infection of the placenta? [19-3a]:3. Potter Syndrome History- This term male infant was born to a mother who had been noted to have oligohydramnios throughout the third trimester. The infant experienced severe respiratory distress and died minutes after birth. A. Note the 'cramped' position of the infant with the arms folded across the chest (compressing the thorax) and the legs bent across the abdomen (twisting the feet). [19-3b]:3B. From the side, the typical features of Potter's Facies can be seen including a sloping forehead, flattened nose, recessed chin and lowset floppy ears (elephant-like). [19-3c]:3C. The opened thorax shows a narrow thoracic cavity occupied mainly by the heart, allowing little room for the development of the lungs. [19-3d]:3D. The lungs, while appearing normal in configuration, weigh only 22 grams vs an expected weight of 54 grams for a term infant. [19-3e]:3E. A section from the periphery of the lungs shows a terminal bronchiole just beneath the pleura. A radial alveolar count (RAC) is made by extending a perpendicular line from the center of the bronchiole to the pleura and counting the number of alveolar septa crossed. In a term infant this number is usually 5 or 6 compared to 2 or 3 in this infant. This decreased RAC is indicative of pulmonary hypoplasia. [19-3f]:3F. With the bowel distal to the stomach removed from the abdomen, the vertebral column and adjacent psoas muscles are easily seen. The kidneys, however, are absent, although the adrenals can be seen near the top of the psoas muscles on either side. [19-3g]:G. The surface of the placenta is studded with small white nodules (amnion nodosum) representing collections of amniotic cells stuck to the surface, a frequent finding in cases of oligohydramnios. [19-3h]:H. While the kidneys in this Potter's Syndrome infant are present, they are markedly dilated and filled with fluid. This congenital hydronephrosis was secondary to an atresia of the urethra. Potter's Syndrome Questions- 1. What is the mechanism of development (i.e., the etiology) of Potter's Syndrome? 2. What are the major features of Potter's Syndrome? 3. What renal anomalies are associated with the development of Potters Syndrome? 4. What are some other causes of pulmonary hypoplasia? [19-4a]:4. Wilms' Tumor - Nephroblastoma History- This five year old boy was noted by his pediatrician on routine physical exam to have a mass in the right lower quadrant of his abdomen. A CT scan displayed a mass virtually replacing the right kidney. The left kidney was unremarkable. The mass was resected. A. The mass is composed of encapsulated pale tan, lobulated tissue subdivided by irregular fibrous septa. Note the thin rim of normal kidney at upper right. [19-4b]:4B. The tumor consist of sheets and clusters of 'small round blue cells' separated by bands of fibrous connective tissue. Note the duct-like structures within the 'blastemal' cell clusters. [19-4c]:4C. In some areas the entire tumor is composed of tubular structures . [19-4d]:4D. In other areas mesenchymal components resembling immature fibroblasts predominate. [19-4e]:4E. Within the blastemal area clusters of large, hyperchromatic cells with bizarre mitoses (anaplasia) may be present. [19-4f]:4F. Spread of tumor may be seen along veins in the renal hilus. Questions 1. What are the anomalies and syndromes associated with Wilms' tumor? 2. What are the tissue patterns or cells types noted in Wilms' tumor? 3. What are the good and bad prognostic indicators of Wilms' tumor? [19-5a]:5. Rhabdomyosarcoma History- This 9 year old male complained of constipation, and on physical examination was note to have a large retroperitoneal mass that compressed the rectum. The mass was excised. A. The mass is poorly encapsulated and composed of irregular nodules of tan-white tissue. [19-5b]:5B. On cut section the mass consists of soft, tan tissue subdivided by fibrous septa. [19-5c]:5C. In the embryonal form of rhabdomyosarcoma, the tumor is composed of sheets of round to tapered cells in a rich vascular stroma. [19-5d]:5D. In this embryonal rhabdomyosarcoma, note the elongate cells with 'tails' of granular cytoplasm (strap cells) and plump cells with abundant cytoplasm (rhabdomyoblasts). Cross striations may occasionally be seen in these cells. [19-5e]:5E. The alveolar pattern of rhabdomyosarcoma consists of fibrous septa lined by or covered with 'small round blue cells' that may fill the intervening spaces. [19-5f]:5F. The individual cells of the alveolar form may have eccentric collections of eosinophilic cytoplasm (rhabdomyoblasts). [19-5g]:5G. When arising in a hollow structure (bladder, vagina, bile duct) a rhabdomyosarcoma forms 'grape-like' structures and is referred to as botryoid ( a gross description only). [19-5h]:5H. In a botryoid rhabdomyosarcoma, the embryonal pattern consists of a dense collection of rhabdomyoblasts beneath the normal epithelium of the structure involved. Deeper to the epithelium, the cells are less dense and strap cells with cross striations may be present. Questions- 1. What are the most common sites of involvement of rhabdomyosarcoma in children? 2. What are the two basic tissue patterns of rhabdomyosarcoma in children? 3. What factors influence the prognosis of rhabdomyosarcoma?
Revista Española De Patología 1982; 15 (4, Octubre-Diciembre) nephroblastoma. Histologic pattern in relation with the natural history ofthe disease, 337346. I. Peculiarities. II. congenital tumors, 347-363. http://www.repatologia.com/crearficheros/1982_4_en.html
Extractions: Nephroblastoma. Histologic pattern in relation with the natural history of the disease Contreras F, Picazo MªL, Suárez A Ciudad Sanitaria "La Paz". Madrid Childhood tumors of the central nervous system. I. Peculiarities. II. Congenital tumors Cusi Sánchez V, Vila Torres J, Royo Sanz C Hospital Infantil San Juan de Dios de Barcelona Palabras Clave: brain tumors in infancy, congenital tumors, calcifications
ICON Health Publications -- Home Page lymphoma hodgkin's, child malignant lymphoma, child nephroblastoma, child rhabdosarcoma atrophy,cirrhosis of the liver, cirrhosis, congenital pigmentary, clam http://www.icongrouponline.com/health/healthC.html
FamilyFun: Health Encyclopedia: Wilm's Tumor nephroblastoma, also known as Wilms' tumor, is the most common kidney cancer in Oneof six children who develop the malignancy has congenital deformities of http://familyfun.go.com/raisingkids/child/health/childhealth/dony89enc_wilm/
Extractions: Nephroblastoma, also known as Wilms' tumor, is the most common kidney cancer in children. The tumor usually appears in children less than five years of age (the median age is three), although it may develop in adolescence. Wilms' tumor is about 90 percent curable. Even if the cancer has spread to distant organs, more than half of all patients survive. WHAT CAUSES WILMS' TUMOR? The tumor sometimes runs in families, indicating a genetic influence yet to be defined. One of six children who develop the malignancy has congenital deformities of the urinary tract of the genitals (such as a malformation of the urethra or an undescended testicle ). Other associated abnormalities include structural deformities of the heart and absence of the iris from the eye. Wilms' tumor sometimes develops in a child who has relatives with these congenital abnormalities.
UNSW Embryology-OMIM WILMS TUMOR 1 nephroblastoma. The syndrome of aniridia, hemihypertrophy and other congenital anomalieswith Wilms tumor, subsequently known as the WAGR syndrome, was first http://anatomy.med.unsw.edu.au/cbl/embryo/OMIMfind/kidney/OMIM-194070.htm
ÅÔÁÉÑÅÉÁ ÉÁÔÑÉÊÙÍ ÓÐÏÕÄÙÍ - ÂÇÔÁ ÉÁÔÑÉÊÅÓ Å ABSTRACT nephroblastoma or Wilms' tumor accounts for most childhood renal neoplasms featureof Wilms' tumor is its association with congenital anomalies (eg http://www.mednet.gr/beta/98-8.htm
Extractions: Dept. of Biology and Genetics, Larisa University, School of Medicine, Larisa, Greece ABSTRACT Childhood osteoporosis is a rather rare disease affecting normal bone growth, development and homeostasis. This review summarizes the most common diagnostic methods, symptomatology, complications and genetic and other etiologic predisposing factors of the disease. In addition, it is well known that various endocrine and metabolic diseases contribute to the development of childhood osteoporosis. In this respect, their specific therapeutic correction appears to result in the proper management of osteoporotic symptoms, as well. While hormone replacement therapy in hormone deficient children can effectively prevent osteoporosis, equally effective therapeutic approaches for the other forms of the disease are presently lacking. Future advances in biomedicine, including the use of growth factors and somatic cell gene therapy, may help ameliorate or extinguish the devastating effects of this highly debilitating disease.
Service Page - Pathologie Information nephroblastoma or Wilms tumor is the most frequent renal tumor in children nephroblastomasmay be associated with a variety of congenital malformations (aniridia http://orphanet.infobiogen.fr/Site/Exp.stm?Lng=GB&Expert=654
Extractions: References Denys et al. and Drash et al. were the first to report the triad of progressive renal disease, male pseudohermaphroditism and Wilms' tumor [1,2]. All of the patients were infants with severe proteinuria progressing rapidly to renal failure. Incomplete forms of the syndrome were described and the glomerulopathy was identified as diffuse mesangial sclerosis (DMS) [3]. During the early stages, the glomerular lesions are characterized by a high amount of fibrils in the mesangial matrix without mesangial cell proliferation [4-6]. The capillary walls are lined with hypertrophied podocytes. The fully developed lesion consists of thickened glomerular basement membranes associated with massive enlargement of mesangial areas, leading to the reduction of the capillary lumens. Mesangial sclerosis eventually contracts the glomerular tuft into a sclerotic mass within a dilated urinary space. There is usually a corticomedullary gradient of involvement, with the most central glomeruli being less affected. Tubules are severely damaged, especially in the deeper cortex, where they are markedly dilated and often contain hyaline casts.
PaediatricNursing Paper I & II syndromes, UTI/Renal failure, obstructive uropathy, congenital defects hynospadis Lymphomes,bleeding disorders, brain tumour, nephroblastoma, bonetumuours. http://www.tnpsc.org/PaediatricNurspg1.htm
